Cardiac surgery-associated acute kidney injury is common. In order to improve our understanding of acute kidney injury, we formed the multi-centre Neonatal and Pediatric Heart and Renal Outcomes Network. Our main goals are to describe neonatal kidney injury epidemiology, evaluate variability in diagnosis and management, identify risk factors, investigate the impact of fluid overload, and explore associations with outcomes.

The Neonatal and Pediatric Heart and Renal Outcomes Network collaborative includes representatives from paediatric cardiac critical care, cardiology, nephrology, and cardiac surgery. The collaborative sites and infrastructure are part of the Pediatric Cardiac Critical Care Consortium. An acute kidney injury module was developed and merged into the existing infrastructure. A total of twenty-two participating centres provided data on 100–150 consecutive neonates who underwent cardiac surgery within the first 30 post-natal days. Additional acute kidney injury variables were abstracted by chart review and merged with the corresponding record in the quality improvement database. Exclusion criteria included >1 operation in the 7-day study period, pre-operative renal replacement therapy, pre-operative serum creatinine >1.5 mg/dl, and need for extracorporeal support in the operating room or within 24 hours after the index operation.

A total of 2240 neonatal patients were enrolled across 22 centres. The incidence of acute kidney injury was 54% (stage 1 = 31%, stage 2 = 13%, and stage 3 = 9%).

Neonatal and Pediatric Heart and Renal Outcomes Network represents the largest multi-centre study of neonatal kidney injury. This new network will enhance our understanding of kidney injury and its complications.

The air gap technique (AGT) is an approach to radiation dose optimisation during fluoroscopy where an “air gap” is used in place of an anti-scatter grid to reduce scatter irradiation. The AGT is effective in adults but remains largely untested in children. Effects are expected to vary depending on patient size and the amount of scatter irradiation produced.

Fluoroscopy and cineangiography were performed using a Phillips Allura Fluoroscope on tissue simulation anthropomorphic phantoms representing a neonate, 5-year-old, and teenager. Monte Carlo simulations were then used to estimate effective radiation dose first using a standard recommended imaging approach and then repeated using the AGT. Objective image quality assessments were performed using an image quality phantom.

Effective radiation doses for the neonate and 5-year-old phantom increased consistently (2–92%) when the AGT was used compared to the standard recommended imaging approaches in which the anti-scatter grid is removed at baseline. In the teenage phantom, the AGT reduced effective doses by 5–59%, with greater dose reductions for imaging across the greater thoracic dimension of lateral projection. The AGT increased geometric magnification but with no detectable change in image blur or contrast differentiation.

The AGT is an effective approach for dose reduction in larger patients, particularly for lateral imaging. Compared to the current dose optimisation guidelines, the technique may be harmful in smaller children where scatter irradiation is minimal.

There are several agents used for conscious sedation by various routes in children. The aim of this prospective randomised study is to compare the effectiveness of three commonly used sedatives: intranasal ketamine, intranasal midazolam, and oral chloral hydrate for children undergoing transthoracic echocardiography.

Children who were referred to paediatric cardiology due to a heart murmur for transthoracic echocardiography were prospectively randomised into three groups. Seventy-three children received intranasal midazolam (0.2 mg/kg), 72 children received intranasal ketamine (4 mg/kg), and 72 children received oral chloral hydrate (50 mg/kg) for conscious sedation. The effects of three agents were evaluated in terms of intensity, onset, and duration of sedation. Obtaining high-quality transthoracic echocardiography images (i.e. absence of artefacts) were regarded as successful sedation. Side effects due to medications were also noted.

There was no statistical difference in terms of sedation success rates between three groups (95.9, 95.9, and 94.5%, respectively). The median onset of sedation in the midazolam, ketamine, and chloral hydrate was 14 minutes (range 7–65), 34 minutes (range 12–56), and 40 minutes (range 25–57), respectively (p < 0.001 for all). However, the median duration of sedation in study groups was 68 minutes (range 20–75), 55 minutes (range 25–75), and 61 minutes (range 34–78), respectively (p = 0.023, 0.712, and 0.045). Gastrointestinal side effects such as nausea and vomiting were significantly higher in the chloral hydrate group (11.7 versus 0% for midazolam and 2.8% for ketamine, respectively, p = 0.002).

Results of our prospectively randomised study indicate that all three agents provide adequate sedation for successful transthoracic echocardiography. When compared the three sedatives, intranasal midazolam has a more rapid onset of sedation while intranasal ketamine has a shorter duration of sedation. Intranasal ketamine can be used safely with fewer side effects in children undergoing transthoracic echocardiography.

Cardiovascular disease is a leading cause of morbidity and mortality in childhood cancer survivors. Cardiologists must be aware of risk factors and long-term follow-up guidelines, which have historically been the purview of oncologists. Little is known about paediatric cardiologists’ knowledge regarding the cardiotoxicity of cancer treatment and how to improve this knowledge.

A total of 58 paediatric cardiologists anonymously completed a 21-question, web-based survey focused on four cardio-oncology themes: cancer treatment-related risk factors (n = 6), patient-related risk factors (n = 6), recommended surveillance (n = 3), and cardiac-specific considerations (n = 6). Following the baseline survey, a multi-disciplinary team of paediatric cardiologists and cancer survivor providers developed an in-person and web-based educational intervention. A post-intervention survey was conducted 5 months later.

The response rate was 41/58 (70.7%) pre-intervention and 30/58 (51.7%) post-intervention. On the baseline survey, the percentage of correct answers was 68.8 ± 10.3%, which improved to 79.2 ± 16.2% after the intervention (p = 0.009). The theme with the most profound knowledge deficit was surveillance; however, it also had the greatest improvement after the intervention (49.6 ± 26.7 versus 66.7 ± 27.7% correct, p = 0.025). Individual questions with the largest per cent improvement pertained to risk of cardiac dysfunction with time since treatment (52.4 versus 93.1%, p = 0.002) and the role of dexrazoxane (48.8 versus 82.8%, p = 0.020).

Specific knowledge deficits about the care of paediatric cancer survivors were identified amongst cardiologists using a web-based survey. Knowledge of surveillance was initially lowest but improved the most after an educational intervention. This highlights the need for cardio-oncology-based educational initiatives among paediatric cardiologists.

In this study, we applied ultra-short time series of interbeat intervals (RR-intervals) to evaluate heart rate variability through default chaotic global techniques with the purpose of discriminating obese youths from non-obese youth patients.

Chaotic global analysis of the RR-intervals from the electrocardiogram and pre-processing adjustments was undertaken. The effect of cubic spline interpolations was assessed, while the spectral parameters remained fixed. Exactly, 125 RR-intervals of data were recorded.

CFP1, CFP3, and CFP6 were the only significant combinations of chaotic globals when the standard conditions were enforced and at the level p<0.01 (or <1%). These significances were acheived via Kruskal–Wallis and Cohen’s ds effects sizes tests of significance after Anderson–Darling and Lilliefors statistical tests indicated non-normal distributions in the majority of cases. Adjustments of the cubic spline interpolation from 1 to 13 Hz were revealed to be inconsequential when measured by Kruskal–Wallis and Cohen’s ds, regarding the outcome between the two datasets.

Chaotic global analysis was offered as a robust technique to distinguish autonomic dysfunction in obese youths. It can discriminate the two different groups using ultra-short data lengths, and no cubic spline interpolations need be applied.

Some authors advocate the use of a dedicated formula to predict the Fontan pressure starting from pre-Fontan catheterisation data. This paper aims at testing the predictive value of the mentioned formula through a retrospective clinical study.

Pre-Fontan catheterisation data and Fontan pressure measured at the completion were retrospectively collected. Pre-Fontan data were used to calculate the predicted pressure in the Fontan system. The predicted values were compared to the Fontan pressure measured at the Fontan completion and with the needs for fenestration. One hundred twenty-four Fontan patients were retrospectively enrolled (At Fontan: median age 30.73 [24.70–37.20] months, median weight 12.00 [10.98–14.15] kg). Fontan conduit was fenestrated in 78 patients. A poor correlation (r2 = 0.05128) between the measured and predicted data for non-fenestrated patients was observed. In the case of Fontan-predicted pressure <17.59 mmHg, the formula identified a good short-term clinical outcome with a sensitivity of 92%.

The proposed formula showed a poor capability in estimating the actual pressure into the Fontan system and in identifying patients needing fenestration. As the pressure into the Fontan system is determined by multiple factors, the tested formula could be an additional data in a multi-parametric approach.

Anthracycline chemotherapeutic agents carry the well-recognised risk of cardiac toxicity. The aim of this study was to determine the long-term effect of anthracycline chemotherapy on the biventricular function in childhood cancer survivors using tissue Doppler imaging and two-dimensional speckle tracking echocardiography.

The study included 45 survivors of childhood cancers and 50 healthy age-matched control patients. Cardiac function was prospectively studied with conventional echocardiography, tissue Doppler imaging, and speckle tracking echocardiography after completion of treatment. The same analysis was performed on matched controls.

There was no difference in age, gender, height, and weight between the study and control groups. The mean anthracycline dose was 240 ± 106 mg/m2 and the mean remission duration was 8.2 ± 5 years (1–20 years) in the study group. Conventional echocardiography showed similar ejection fraction, shortening fraction, and left ventricle end-diastolic diameter in both groups. Mitral lateral and septal tissue Doppler imaging showed normal but according to control group relatively sub-normal systolic and diastolic function in patient group. The global longitudinal and circumferential strain and strain rates were significantly lower in the patient group compared to control group. Correlation analysis revealed a negative and significant correlation between total anthracycline dose and global longitudinal and circumferential strain and strain rates.

Sub-clinical systolic and diastolic dysfunction may not be detected by conventional echocardiographic methods which are frequently used in daily practice. Sub-clinical systolic and diastolic dysfunction may be detected more sensitively by echocardiographic method such as speckle tracking echocardiography in childhood cancer survivors.

To analyse the relationship of altered birth weight with metabolic and cardiovascular outcomes among adolescents, as well as to identify if sports participation is able to attenuate or even eliminate the impact of birth weight on health outcomes.

Cross-sectional study (Analysis of Behaviours of Children During Growth [ABCD Growth Study]). Adolescents with age ranging from 11 to 18 years old (14.7±2.1) stratified according to normal (n = 230) and altered (n = 35) birth weight composed the sample. Birth weight was self-reported by adolescent’s parents. Sports participation was assessed by face-to-face interview. Carotid intima–media thickness (CIMT) and femoral intima–media thickness (FIMT) were measured using an ultrasound device. C-reactive protein levels were used to assess the inflammatory status. Blood pressure, Z score of metabolic risk (dyslipidemia and glucose), adiposity, and insulin resistance were covariates.

In the crude model, FIMT (p value = 0.037) and C-reactive protein (p value = 0.029) were affected by altered birth weight. In the adjusted models, altered birth weight affected FIMT (p value = 0.048; small effect size of 1.7%), independently of sports participation. For C-reactive protein, previous time of engagement in sports (p value = 0.001; small effect size of 4.8%) affected C-reactive protein, independently of birth weight.

Vascular structure seems to be affected by birth weight in adolescents, while its impact on inflammation seems to be attenuated by the regular engagement in sports.

Ductal patency is mandatory to manage patients with ductal-dependent pulmonary circulation. The aim of this study is to elucidate the morphological and haemodynamic features of ductus arteriosus with right ventricular outflow tract obstruction, and investigate the appropriate perinatal management.

Patients with prenatal diagnosis of right ventricular outflow tract obstruction at our institution between 2010 and 2015 were included in the study. Reverse orientation of the ductus arteriosus is defined as an inferior angle of <90° at the aortic junction, and normal orientation of the ductus arteriosus as an angle of >90°. We retrospectively reviewed the shape and flow pattern of ductus arteriosus and the clinical characteristics of the cases.

A total of 39 patients were enrolled. The shape was divided into normal orientation (n=15) and reverse orientation (n=24) of the ductus arteriosus. There was no significant difference in the type of oxygen saturation at birth and age at shunt operation between both the groups. However, the median narrowest diameter of ductus arteriosus in the normal orientation group was significantly smaller than that in the reverse orientation group (2.0 [1.0–5.4] versus 3.0 [1.3–4.4] mm, p<0.05). In two patients of the normal orientation group, ductus arteriosus had closed at birth, and one of whom died because of severe cyanosis.

Normal orientation pattern might have high incidence of an early narrowing or closure of ductus arteriosus at birth. The critical patients need careful evaluation by repeated foetal echocardiography and further maternal interventions.

Prenatal diagnosis and planned peri-partum care is an unexplored concept for care of neonates with critical CHDs in low-middle-income countries.

To report the impact of prenatal diagnosis on pre-operative status in neonates with critical CHD.

Prospective observational study (January 2017–June 2018) in tertiary paediatric cardiac facility in Kerala, India. Neonates (<28 days) with critical CHDs needing cardiac interventions were included. Pre-term infants (<35 weeks) and those without intention to treat were excluded. Patients were grouped into those with prenatal diagnosis and diagnosis after birth. Main outcome measure was pre-operative clinical status.

Total 119 neonates included; 39 (32.8%) had prenatal diagnosis. Eighty infants (67%) underwent surgery while 32 (27%) needed catheter-based interventions. Pre-operative status was significantly better in prenatal group; California modification of transport risk index of physiological stability (Ca-TRIPS) score: median 6 (0–42) versus 8 (0–64); p < 0.001; pre-operative assessment of cardiac and haemodynamic status (PRACHS) score: median 1 (0–4) versus 3 (0–10), p < 0.001. Age at cardiac procedure was earlier in prenatal group (median 5 (1–26) versus 7 (1–43) days; p = 0.02). Mortality occurred in 12 patients (10%), with 3 post-operative deaths (2.5%). Pre-operative mortality was higher in postnatal group (10% versus 2.6%; p = 0.2) of which seven (6%) died due to suboptimal pre-operative status precluding surgery.

Prenatal diagnosis and planned peri-partum care had a significant impact on the pre-operative status in neonates with critical CHD in a low-resource setting.

The present study aimed to evaluate systolic and diastolic myocardial function in children and adolescents with congenital adrenal hyperplasia.

The study included 44 children with the diagnosis of classic congenital adrenal hyperplasia and 39 healthy children whose age, pubertal status, and gender were similar to those of the patient group. Anthropometric parameters and 17-hydroxyprogesterone levels were measured, and bone age was calculated. The average daily hydrocortisone dose was calculated over the last 1-year file records. Hyperandrogenic state was defined according to bone age SD score (⩾2) and 17-hydroxyprogesterone levels (>10 ng/ml). Echocardiographic examinations were assessed by conventional two-dimensional Doppler echocardiography and tissue Doppler imaging.

Patients had higher morphological parameters, such as left ventricular end-systolic diameter, interventricular septal thickness at end diastole, left ventricular posterior wall thickness at end diastole, left ventricular mass and index, than the control group (p<0.05). On pulsed-wave and tissue Doppler echocardiography, significant subclinical alterations were observed in systolic (isovolumic contraction time), diastolic (isovolumic relaxation time), and global left ventricular functional (myocardial performance index) parameters in the congenital adrenal hyperplasia group compared to the control group (p<0.05). In partial correlation analyses, after controlling the effect of hyperandrogenism, the mean hydrocortisone dosage was positively correlated with isovolumic relaxation time in congenital adrenal hyperplasia group (p<0.05).

This study demonstrated that the patients with congenital adrenal hyperplasia are at risk for left ventricular hypertrophy, systolic and diastolic myocardial subclinical alterations. Overtreatment may be responsible for the increased risk of myocardial dysfunction in patients with congenital adrenal hyperplasia.

Although percutaneous closure of patent ductus arteriosus is an established safe procedure, protrusion of the device to descending aorta may occur in various degrees during these procedures, especially in small infants. The aim of our study is to evaluate the benefits of balloon-assisted device releasing technique in the era of preventing device protrusion and conditions related to protrusion.

One hundred and fifty-five infants, who underwent patent ductus arteriosus closure with Amplatzer duct occluder I device between January, 2012 and December, 2018, were retrospectively analysed. Balloon-assisted device releasing technique was used in 20 cases (group 1, 12.9%), between January, 2015 and December, 2018. Procedures in which the technique had been used were compared with the remaining ones (group 2, 87.1%, n = 135) with regard to device stabilisation, aortic disc protrusion to the aorta, iatrogenic coarctation, and device embolisation.

There was no significant difference by means of gender, age, weight, and the ductal diameter, whereas the average mean pulmonary artery pressure was significantly higher in group 1. Device protrusion and related complications were significantly higher in group 2; thus, additional catheterisations or surgical interventions were required, while no additional intervention was required in group 1.

The balloon-assisted device releasing technique provides a good device stabilisation and prevents protrusion of the device and related complications during percutaneous patent ductus arteriosus closure in selected cases.

The CeraFlexTM PDA occluder is a new flexible device with a unique delivery system that may be beneficial with regard to not changing the device position after releasing. We prospectively evaluate the efficacy of the device and also the device behaviour patterns during release.

The study included 21 patients. Their median age was 1.2 years (from 6 months to 28 years) and weight was 9.6 kg (from 5.4 to 82 kg). All of the ducts were conical except one atypical ductus. Median ductal diameter at the pulmonary end was 3.8 mm (from 2.2 to 8.2 mm). The ductus was closed using an antegrade approach, but special attention was paid to the patterns of device behaviour during and just after releasing.

Three different modes of device behaviour were observed during and just after releasing: (1) Neither difficulty nor change of position in 13 patients (62%), (2) a little difficulty in releasing but no change of position in 6 (29%), and (3) change of the device position in 2 (9%). There was no residual shunt on the next day except in one patient, in whom late device embolisation occurred. The device was retrieved and another, bigger device implanted.

The CeraFlexTM PDA occlude device seems to be safe and efficacious for patent ductus arteriosus closure. Its unique delivery system generally fixes the device in a stable position that does not change after release (91%). Minor difficulty in releasing is not uncommon; however, the major disadvantage is the need for larger sheaths for delivery.

We aimed to describe the profile and treatment outcomes of infant and childhood endocarditis at our facilities.

This is a retrospective analysis of infants and children with endocarditis at two public sector hospitals in the Western Cape Province of South Africa over a 5-year period. Patients with “definite” and “possible” endocarditis according to Modified Duke Criteria were included in the review.

Forty-nine patients were identified for inclusion; 29 had congenital heart disease as a predisposing condition; 64% of patients met “definite” and 36% “possible” criteria. The in-hospital mortality rate was 20%; 53% of patients underwent surgery with a post-operative mortality rate of 7.7%. The median interval from diagnosis to surgery was 20 days (interquartile range, 9–47 days). Valve replacement occurred in 28% and valve repair in 58%. There was a significant reduction in valvular dysfunction in patients undergoing surgery and only a marginal improvement in patients treated medically. Overall, 43% of patients had some degree of residual valvular dysfunction.

Endocarditis is a serious disease with a high in-hospital mortality and presents challenges in making an accurate diagnosis. Despite a significant reduction in valvular dysfunction, a portion of patients had residual valvular dysfunction. Early surgery is associated with a lower mortality rate, but a higher rate of valve replacement compared with delayed surgery.

Atrial septal defect is the third most common CHD. A hemodynamically significant atrial septal defect causes volume overload of the right side of the heart. Preterm children may suffer from both pulmonary and cardiac comorbidities, including altered myocardial function. The aim of this study was to compare the rate of adverse events following atrial septal defect closure in preterm- and term-born children.

We performed a retrospective cohort study including children born in Sweden, who had a surgical or percutaneous atrial septal defect closure at the children’s hospitals in Lund and Stockholm, between 2000 and 2014, assessing time to the first event within 1 month or 1 year. We analysed differences in the number of and the time to events between the preterm and term cohort using the Kaplan–Meier survival curve, a generalised model applying zero-inflated Poisson distribution and Gary-Anderson’s method.

Overall, 413 children were included in the study. Of these, 93 (22.5%) were born prematurely. The total number of adverse events was 178 (110 minor and 68 major). There was no difference between the cohorts in the number of events, whether within 1 month or within a year, between major (p = 0.69) and minor (p = 0.84) events or frequencies of multiple events (p = 0.92).

Despite earlier procedural age, larger atrial septal defects, and higher comorbidity than term children, preterm children appear to have comparable risk for complications during the first year after surgical or percutaneous closure.

Little is known about health-related quality of life in young children undergoing staged palliation for single-ventricle CHD. The aim of this study was to assess the impact of CHD on daily life in pre-schoolers with single-ventricle CHD and to identify determinants of health-related quality of life.

Prospective two-centre cohort study assessing health-related quality of life using the Preschool Paediatric Cardiac Quality of Life Inventory in 46 children at a mean age of 38 months and 3 weeks. Children with genetic anomalies were excluded. Scores were compared with reference data of children with biventricular CHD. Multiple linear regression analysis was used to identify determinants of health-related quality of life.

Health-related quality of life in pre-schoolers with single-ventricle CHD was comparable to children with biventricular CHD. Preterm birth and perioperative variables were significant predictors of low health-related quality of life. Notably, pre-Fontan brain MRI findings and neurodevelopmental status were not associated with health-related quality of life. Overall, perioperative variables explained 24% of the variability of the total health-related quality of life score.

Despite substantial health-related burden, pre-schoolers with single-ventricle CHD showed good health-related quality of life. Less-modifiable treatment-related risk factors and preterm birth had the highest impact on health-related quality of life. Long-term follow-up assessment of self-reported health-related quality of life is needed to identify patients with poorer health-related quality of life and to initiate supportive care.

Myocardial deformation by speckle tracking echocardiography provides additional information on left ventricular function. Values of myocardial deformation (strain and strain rate) depend on the type of ultrasound machine and software that is used. Normative values for QLAB (Philips) are scarce, especially for children. It is important to evaluate the influence of age and body size on myocardial deformation parameters, since anthropometrics strongly influence many standard echocardiographic parameters. The aim of this study was to provide comprehensive normal values for myocardial deformation of the left ventricle using a Philips platform and to evaluate the association with anthropometric and standard echocardiographic parameters.

Healthy children between 1 and 18 years of age were prospectively examined using a standard echocardiographic protocol. Short-axis and apical four-chamber, two-chamber, and three-chamber views were used to measure peak systolic circumferential and longitudinal strain and systolic and early diastolic strain rate of the left ventricle using dedicated software.

A total of 103 children were included with a mean age of 10.8 and inter-quartile range 7.3–14.3 years. Global circumferential strain values (±SD) were −24.2±3.5% at basal, −25.8±3.5% at papillary muscle, and −31.9±6.2% at apex levels. Global left ventricular longitudinal strain values were −20.6±2.6% in apical four-chamber view, −20.9±2.7% in apical two-chamber, and −21.0 ±2.7% in apical three-chamber. Age was associated with longitudinal strain, longitudinal systolic and early diastolic strain rate, but not with circumferential strain.

Normal values for left ventricular deformation parameters in children are obtained using a Philips platform. Age partly explains normal variation of strain and strain rate.